Global coagulation assays in hemophilia A: A comparison to conventional assays.

Background: Global assays measure the interactions of coagulants, anticoagulants, and platelets on thrombin generation and may reflect the comprehensive coagulation potential in patients with hemophilia better than conventional assays.

Objectives: The objectives of the current study were to investigate the value of global assays for measuring and monitoring the coagulation potential of patients with hemophilia A (HA).

Patients/methods: Rotational thromboelastometry, thrombin generation assay (TGA), and activated partial thromboplastin time (APTT) clot waveform analysis were investigated in a cohort of patients with severe, moderate, and mild HA and compared with conventional assays.

Pharmacokinetics, immunogenicity, safety, and preliminary efficacy of subcutaneous turoctocog alfa pegol in previously treated patients with severe hemophilia A (alleviate 1).

Background: The current standard of care for patients with hemophilia A is regular prophylaxis with factor VIII (FVIII) administered intravenously. Interest in subcutaneous (s.c.

A stepwise thrombolysis regimen in the management of acute portal vein thrombosis in patients with evidence of intestinal ischaemia.

Background: Anticoagulation alone in acute, extensive portomesenteric vein thrombosis (PVT) does not always result in spontaneous clot lysis, and leaves the patient at risk of complications including intestinal infarction and portal hypertension.

Aim: To develop a new standard of care for patients with acute PVT and evidence of intestinal ischaemia.

Methods: We present a case series of patients with acute PVT and evidence of intestinal ischaemia plus ongoing symptoms despite initial systemic anticoagulation, who were treated with a thrombolysis protocol between 2014 and 2019.

Anticoagulation after Iliofemoral Vein Stenting - Old Versus New.

In recent years, there has been an increasing interest in endovascular iliofemoral vein stenting to prevent/ alleviate symptoms related to proximal venous outflow obstruction. Maintaining long-term stent patency is one of the main challenges, and risk factors for the development of re-thrombosis are not well understood. Published data on the safety and efficacy of the procedure predominantly come from cohort studies mainly focusing on mechanical aspects relating to stent placement and flow.

Anti-tissue factor pathway inhibitor (TFPI) therapy: a novel approach to the treatment of haemophilia.

Novel approaches to the treatment of haemophilia are needed due to the limitations of the current standard of care, factor replacement therapy. Aspirations include lessening the treatment burden and effectively preventing joint damage. Treating haemophilia by restoring thrombin generation may be an effective approach.

Phase 1, single-dose escalating study of marzeptacog alfa (activated), a recombinant factor VIIa variant, in patients with severe hemophilia.

Unlabelled: Essentials Marzeptacog alfa (activated) [MarzAA] is a novel variant of activated human factor VII. A phase 1 dose escalation trial of MarzAA was conducted in subjects with severe hemophilia. MarzAA was safe and tolerated at intravenous doses up to 30 μg kg Data observed support further trials for hemophilia patients with inhibitors to factors VIII/IX.

Review of recombinant anti-haemophilic porcine sequence factor VIII in adults with acquired haemophilia A.

Acquired haemophilia A (AHA) is a rare, serious bleeding disorder most often encountered in elderly patients. The mainstay of haemostatic management is with bypassing agents (BPAs) including recombinant activated factor VII (rFVIIa) and activated prothrombin complex concentrates (aPCCs). Their major limitation is incomplete efficacy, potential risk for thrombosis and the lack of routine laboratory assays for monitoring treatment response.

Panton-Valentine Leukocidin Producing Staphylococcus Aureus Facial Pyomyositis Causing Partial Cavernous Sinus Thrombosis.

We present a case of subtotal cavernous sinus thrombosis secondary to Panton-Valentine leukocidin-associated Staphylococcus aureus pyomyositis of the muscles of mastication in a previously healthy child, who was successfully managed with no residual disease. He was found to have a factor V Leiden heterozygous mutation. We highlight the propensity of Panton-Valentine leukocidin Staphylococcus aureus to induce venous thrombosis at any site but with potential for more severe consequences in the head.